摘要:

Hereditary deficiency of factor VII is demonstrated in a Chinese family. The proposita was a 32‐year‐old female with bleeding diathesis consisting of spontaneous ecchymosis, menorrhagia and recurrent haemarthrosis. The prothrombin time was prolonged and the prothrombin and proconvertin test was 10% of normal. The activated partial thromboplastin time and the Stypven‐cephalin clotting time were normal. The prolonged prothrombin time could be corrected by the addition of normal serum, but not by adsorbed normal or coumadin plasma. The factor VII level was 3.6% of normal. One of her brothers had bleeding symptoms and died at age 25, suggesting that factor VII deficiency might have been present. 13 of her family members had partial deficiency of factor VII with plasma levels ranging from 24 to 50%. These results suggest an autosomal recessive inheritance with a homozygous state occurring in the proposita and possibly in her brother, and a heterozygous state occurring in 13 of her family members. Our study marks an extensive survey of factor VII deficiency in an Oriental f

ISSN:0036-553X    

DOI:10.1111/j.1600-0609.1983.tb01450.x

出版商:Blackwell Publishing Ltd    

年代:1983

数据来源: WILEY

摘要:

The influence of the spleen on the distribution of blood neutrophils was studied in rats with varying spleen sizes following transfusion of isologous3H‐dT‐labelled neutrophils. The weight of the spleen correlated with the splenic neutrophil pool (r = 0.87, P<0.01). Alterations in spleen size did not significantly change the total blood neutrophil pool, but with increasing splenomegaly circulating and marginal neutrophils were shifted into the splenic pool. With massive splenomegaly, up to 50% of the total blood neutrophils were trapped in the spleen. These findings suggest splenic pooling of neutrophils and a shift of circulating and marginal neutrophils into the spleen proportionate to the splenic tissue pres

ISSN:0036-553X    

DOI:10.1111/j.1600-0609.1983.tb01451.x

出版商:Blackwell Publishing Ltd    

年代:1983

数据来源: WILEY

摘要:

Granulocyte‐monocyte colony forming cell (GM‐CFC) concentration and the proportion of GM‐CFC in DNA synthesis (S) were determined in 43 patients with varying degrees of selective neutropenia, including 5 patients who were normal extremes (2.0–2.5 times; 109neutrophils/1), to study the diagnostic and prognostic significance of clonogenic assays and to determine the response of the committed myeloid stem cell to neutropenia. The proportion of GM‐CFC in S proved to be a more useful parameter than the GM‐CFC concentration. 75% of the patients with greater than 60% GM‐CFC in S returned to normal within 1 month. Patients with less than 20% GM‐CFC in S had at least a 10 times greater incidence of developing a malignant or autoimmune disease than the other 28 patients. The 11 patients with 41–51% in GM‐CFC in S had greater than 1 times; 109neutrophils/1 and no significant clinical problems but all have remained mildly neutropenic for a long period. An indirect relationship existed between the degree of neutropenia and the proportion of GM

ISSN:0036-553X    

DOI:10.1111/j.1600-0609.1983.tb01452.x

出版商:Blackwell Publishing Ltd    

年代:1983

数据来源: WILEY

摘要:

Two brothers with hypogammaglobulinaemia classified as common variable immunodeficiency (CVID) were investigated for distribution of peripheral blood lymphocyte (PBL) subpopulations, DNA synthesis and plaque‐forming cell (PFC) capability of pokeweed mitogen (PWM) activated autologous and allogenic cocultures. Both patients had a decreased absolute number of T cells and normal or elevated levels of surface immunoglobulin (SmIg) bearing cells.Isolated B cells cocultured with autologous or allogeneic 4000 r irradiated T cells responded subnormally to PWM monitored by the3H‐thymidine incorporation in microcultures whereas B cells cocultured with allogeneic untreated normal T cells proliferated normally.PBL from parallel macrocultures of unfractionated or T/B separated patients' cells were not able to produce plaques using a reversed haemolytic protein A assay. Addition of glucocorticoid to unfractionated PBL did not reverse the unresponsiveness. In allogeneic co‐cultures patients' untreated or 2000 r irradiated T cells induced a normal PFC response. Normal untreated T cells induced a reduced number of IgM‐ and IgG‐PFC from patients' B cells but this response was almost eliminated using irradiated normal T cells.These results demonstrate a primary B cell defect in the patients and indicate an impaired cooperation between patients' B and T cells. Activation of patients' B cells to Ig secretion requires the presence of proliferatin

ISSN:0036-553X    

DOI:10.1111/j.1600-0609.1983.tb01455.x

出版商:Blackwell Publishing Ltd    

年代:1983

数据来源: WILEY

摘要:

Plasma and red cell folate mean contents have been found to be significantly lower in 41 symptom‐free β‐thalassaemia heterozygotes than in 21 controls. Such decreases must be considered as effects of an increased folate utilization caused by the enhanced total, both effective and ineffective, erythropoiesis.Since no close correlation has been found between packed cell volume and plasma or red blood cell folate levels, it seems that in ‘healthy’ β‐thalassaemia subjects, the degree of anaemia is not influenced by folate body reserves. However, the frequent finding of reduced red cell folate contents suggests that further folate imbalance might lead to clinically significant degrees of folate

ISSN:0036-553X    

DOI:10.1111/j.1600-0609.1983.tb01456.x

出版商:Blackwell Publishing Ltd    

年代:1983

数据来源: WILEY

摘要:

Colony formation by haematopoietic progenitors from the bone marrow and blood of 4 patients with essential thrombocythaemia was studied in vitro using the methyl cellulose assay. 3 patients had clearly elevated numbers of BFU‐E in bone marrow. 1 patient also had markedly increased numbers of CFU‐E and CFU‐GM, whereas the other patients had only marginally increased or normal numbers of these progenitors in the marrow. 3 patients showed markedly increased numbers of all progenitors in peripheral blood. All 4 patients showed spontaneous erythroid colony formation by progenitors from the bone marrow and 2 had spontaneous colony formation by progenitors from the blood. We conclude that essential thrombocythaemia shows abnormal colony formation in line with other myeloproliferative synd

ISSN:0036-553X    

DOI:10.1111/j.1600-0609.1983.tb01457.x

出版商:Blackwell Publishing Ltd    

年代:1983

数据来源: WILEY

摘要:

The unsaturated vitamin B12binding capacity (UBBC) of serum, the three transcobalamins (TC I, TC II, TC III), and the total leucocyte and neutrophil counts have been studied in paediatric patients with sickle cell disease (SCD). Increase in the level of the binding capacities of TC I and TC II with concomitant increase of UBBC was observed in these children who also had increased total white blood and neutrophil counts.There was a significant reduction in the level of endogenous B12.These abnormalities are discussed in relation to the deficiency of the splenic reticuloendothelial function, immunologic defect, hepatic degenerative changes and aplastic crisis observed in SCD. These results lend support to the view that transcobalamins are involved in the defence mechanism of the body.The significant reduction in serum cobalamin in SCD suggests a higher demand on this vitamin for metabolic functions, especially for the sparing of folate.

ISSN:0036-553X    

DOI:10.1111/j.1600-0609.1983.tb01459.x

出版商:Blackwell Publishing Ltd    

年代:1983

数据来源: WILEY

摘要:

The thymidine kinase isoenzyme profile was determined in peripheral blood mononuclear cells and splenic tissue from 4 patients with hairy cell leukaemia, in order to assess the proliferative state of the hairy cell. The predominance of TK1 activity in all 4 spleens and in 2 out of 3 peripheral blood mononuclear cells examined, indicates that the hairy cell has significant proliferative capacity when compared to the neoplastic cell in other chronic lymphoproliferative disorders. It is suggested, in viev of the heterogeneity in peripheral blood mononuclear TK isoenzyme types, that more extensive studies are warranted to examine the relationship between peripheral blood mononuclear TK1 activity and the occurrence of progressive disease in post‐splenectomy patient

ISSN:0036-553X    

DOI:10.1111/j.1600-0609.1983.tb01460.x

出版商:Blackwell Publishing Ltd    

年代:1983

数据来源: WILEY

摘要:

Megakaryoblasts and maturing megakaryocytic precursor cells from 5 patients with megakaryoblastic leukaemia were studied by scanning electron microscopy (SEM). The diagnosis in all cases had been established by ultrastructural cytochemistry on the basis of a positive platelet peroxidase reaction with negative staining for myeloperoxidase. 1 case presented as acute myelofibrosis and 4 as acute megakaryoblastic transformation of chronic granulocytic leukaemia. Under the SEM, megakaryoblasts and maturing megakaryocytic precursors showed typical surface features including the presence of rounded and irregular blebs, broad folds and pseudopodia. The nature of these surface blebs is still unclear but they probably represent surface membrane alterations relating to imminent platelet shedding at least in the more mature precursors. These surface microprojections are distinctly different from those encountered on leukaemic lymphoblasts, myeloblasts and monoblasts. It is suggested that SEM may be used in conjunction with the PPO reaction as in aid in the diagnosis of megakaryocytic leukaemias.

ISSN:0036-553X    

DOI:10.1111/j.1600-0609.1983.tb01462.x

出版商:Blackwell Publishing Ltd    

年代:1983

数据来源: WILEY

摘要:

The early in vivo distribution of111Indium‐labelled granulocytes, recorded by dynamic imaging using a gamma camara and computer, varied according to the separation and labelling technique. Following i.v. bolus injection, 4 kinetic patterns could be identified: (A) rapid transit through the pulmonary vasculature, (B) delayed transit through the lung with clearance by about 30 min, (C) complete retention by the lung, for up to 10 min, followed by slow release over a period of 1 to 2 h, (D) delayed transit through the lung with a similar time course to (B) but with subsequent heavy liver uptake.Granulocytes labelled with111In‐tropolonate and maintained in plasma throughout the labelling procedure, whether injected as a ‘pure’ (separated by plasma‐enriched density gradient centrifugation) or ‘crude’ (separated by differential centrifugation) preparation, displayed type A kinetics, thought to most closely represent the normal behaviour of granulocytes. ‘Crude’ cells labelled in saline with111In‐acetylacetonate displayed type B kinetics. ‘Pure’ cells isolated on Percoll‐saline and labelled in saline with111In‐acetyl‐acetonate displayed type C kinetics, thought to represent granulocyte ‘stimulation’ and/or damage, or type D kinetics, thought to represent severe damage. The importance is stressed of labelling granulocytes for kinetic studies with a technique that results in min

ISSN:0036-553X    

DOI:10.1111/j.1600-0609.1983.tb01463.x

出版商:Blackwell Publishing Ltd    

年代:1983

数据来源: WILEY